Elsevier

Current Problems in Cancer

Volume 39, Issue 4, July–August 2015, Pages 258-263
Current Problems in Cancer

Cutaneous angiosarcoma

https://doi.org/10.1016/j.currproblcancer.2015.07.007Get rights and content

Introduction

Angiosarcomas are rare and aggressive tumors of vascular or lymphatic origin representing 1%-2% of all soft tissue sarcomas.1, 2 As a group they exhibit remarkable clinical heterogeneity, occurring in any location of the body. The most common presentation is cutaneous lesion,3 most often on the face and scalp,1, 4, 5 Although surgical resection followed by adjuvant radiotherapy remains the optimal treatment, there is a high risk of locoregional recurrence and many patients progress to disseminated disease. Cytotoxic chemotherapy with anthracycline-, taxane-, and ifosfamide-based regimens produces partial responses in the metastatic setting, but long-term survival remains poor. Targeted therapy using inhibitors of the vascular epidermal growth factor (VEGF) receptor (VEGFR) and broad-spectrum tyrosine kinase inhibitors has recently been reported to produce a clinical response and improve short-term outcomes. The goal of this review is to summarize the current data regarding cutaneous angiosarcoma and its clinical presentation and management.

Section snippets

Clinical presentation

Although soft tissue angiosarcomas have a similar distribution in sexes and can develop at any age, cutaneous angiosarcoma is most common in elderly adult men.3, 6 Most lesions presenting on the face and scalp are sporadic whereas cutaneous angiosarcoma presenting in other locations can be associated with previous radiation therapy or chronic lymphedema. The link between radiotherapy and angiosarcoma was first reported in 1981, when angiosarcoma was observed in the mammary skin of a woman years

Pathologic diagnosis, staging, and prognosis

The histologic features of cutaneous angiosarcoma range from well-differentiated tumors that can mimic benign vascular lesions (atypical vascular lesions) to poorly differentiated tumors that are difficult to distinguish from other cutaneous malignancies such as poorly differentiated melanoma. Well-differentiated tumors consist of irregular interanastomosing channels that infiltrate surrounding tissue. The distinction between angiosarcoma and an atypical vascular lesion hinges on the lack of a

Treatment of localized disease

Radical resection remains the primary treatment of choice for cutaneous angiosarcoma. Positive microscopic (R1) or macroscopic (R2) margins are common because of the invasive and multifocal nature of the disease.1, 3, 5, 6 Wide margins can be difficult to achieve because of intimate relationships with key anatomical structures, especially in the head and neck or in the presence of multifocal disease. Owing to the extensive nature of the disease, resection often results in large surgical defects

Metastatic disease

Cytotoxic chemotherapy with anthracyclines, ifosfamide, and taxanes is the primary systemic treatment option for metastatic cutaneous angiosarcoma. Many angiosarcoma patients are elderly, so comorbidities and the risk of treatment-related toxicity often limits the use of chemotherapy.3 In retrospective series with a variety of chemotherapy regimens, a palliative response can be observed in up to 65% of patients.2, 23, 24, 25, 26 Fury et al reported a series of 52 patients with angiosarcomas

Conclusion

Cutaneous angiosarcoma is a rare and aggressive malignancy with high rates of locoregional and distant recurrence. Surgical resection of gross disease with wide-field adjuvant radiation therapy remains the optimal treatment. Despite aggressive locoregional therapy, many patients progress to disseminated disease. Cytotoxic chemotherapy regimens based on anthracyclines, taxanes, and ifosfamide are used for systemic disease. Emerging data suggest a role for targeted therapy with agents directed at

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References (29)

  • C.A. Holden et al.

    Angiosarcoma of the face and scalp, prognosis and treatment

    Cancer

    (1987)
  • T.M. Pawlik et al.

    Cutaneous angiosarcoma of the scalp: a multidisciplinary approach

    Cancer

    (2003)
  • J. Hamels et al.

    Cutaneous angiosarcoma arising in a mastectomy scar after therapeutic irradiation

    Bull Cancer

    (1981)
  • J. Huang et al.

    Increased risk of soft tissue sarcoma after radiotherapy in women with breast carcinoma

    Cancer

    (2001)
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